MGME1 Antibody / Mitochondrial genome maintenance exonuclease 1

Mitochondrial genome maintenance exonuclease 1 is an enzyme that in humans is encoded by the MGME1 gene. The protein encoded by this gene is a nuclear-encoded mitochondrial protein necessary for the maintenance of mitochondrial genome synthesis. The encoded protein is a RecB-type exonuclease and primarily cleaves single-stranded DNA. Defects in this gene have been associated with mitochondrial DNA depletion syndrome-11. Three transcript variants encoding different isoforms have been found for this gene.